Gardner's syndrome

Gardner's syndrome

[gahrd´nerz]

familial polyposis of the colon associated with osseous and soft tissue tumors.

Gardner's syndrome

Etymology: Eldon J. Gardner, American geneticist, b. 1909

familial polyposis of the large bowel, with fibrous dysplasia of the skull, extra teeth, osteomas, fibromas, and epidermal cysts. The condition is inherited as an autosomal-dominant trait, and malignancies occur more often than usual in families having this syndrome.

It is composed of colonic disorders as colonic polyps which show high potential for malignant transformation and extra colonic disorders such as osteoma, desmoid tumors, and epidermoid cysts which are benign tumors, Fortunately, osteomas and skin manifestations precede polyposis, and this facilitates early diagnosis of Gardner's syndrome and gives chance for rapid prophylactic surgical resection of colonic polyps, which is critical and lifesaving for patients with Gardner's syndrome.

Absence of colorectal polyps, skeletal abnormalities and multiple impacted or supernumerary teeth in this case, was against labelling it as Gardner's syndrome.

However it is expected of a vigilant medical professional that he actively rule out the possibility of Gardner's syndrome as a simple osteoma could be the earliest sign that heralds this syndrome which ultimately manifests as malignant polyps in gastrointestinal system.

Multiple osteomas of skull and jaw bones along with multiple epidermoid sebaceous cysts of skin, desmoids tumors, the major characteristics of gardner's syndrome were also not associated with this case.

Gardner's syndrome: Gardner's syndrome refers to the clinical triad of adenomatous polyps of the gastrointestinal tract with a high risk of malignant transformation, epidermal cysts and dermoid tumors.

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